scholarly journals Improved survival of patients with aggressive ATL by increased use of allo-HCT: A prospective observational study

2021 ◽  
Author(s):  
Ayumu Ito ◽  
Nobuaki Nakano ◽  
Takashi Tanaka ◽  
Shigeo Fuji ◽  
Junya Makiyama ◽  
...  
Keyword(s):  
Observational Study ◽  
Cord Blood ◽  
Treatment Outcomes ◽  
Prospective Observational Study ◽  
Hematopoietic Cell Transplantation ◽  
Curative Therapy ◽  
Adult T Cell Leukemia ◽  
Unrelated Donors ◽  
Multicenter Prospective Observational Study ◽  
Clinical Trials Registry

Aggressive adult T-cell leukemia/lymphoma (ATL) is a hematological malignancy that is difficult to treat with chemotherapy alone, and allogeneic hematopoietic cell transplantation (allo-HCT) is a potentially curative therapy. We conducted a multicenter, prospective, observational study to clarify the treatment outcomes of aggressive ATL in the current era. Between 2015 and 2018, 113 patients aged 70 years or younger with newly diagnosed aggressive ATL were enrolled. The median age at diagnosis was 61 years old. Treatment outcomes were compared with those of 1,792 ATL patients diagnosed between 2000 and 2013 in our previous retrospective study. The inclusion criteria were the same in both studies. The prospective cohort demonstrated better overall survival (OS) than the retrospective cohort (2-year OS, 45% versus 29%, respectively; P<0.001), with a much higher proportion of patients receiving allo-HCT (80% versus 34%, respectively; P<0.001) and a shorter interval from diagnosis to allo-HCT (median, 128 versus 170 days, respectively; P<0.001). Among the 90 patients who received allo-HCT (cord blood, n=30; HLA-haploidentical related donors, n=20; other related donors, n=14; other unrelated donors, n=26), the 2-year probabilities of OS, non-relapse mortality (NRM), and disease progression were 44%, 23%, and 46%, respectively. OS and NRM did not differ statistically according to donor type. Our results suggest that increased application of allo-HCT improved the survival of patients with aggressive ATL. The use of cord blood or HLA-haploidentical donors may be feasible for aggressive ATL when HLA-matched related donors are unavailable. This study was registered with the UMIN Clinical Trials Registry (UMIN 000017672).

scholarly journals Mogamulizumab for adult T-cell leukemia-lymphoma: a multicenter prospective observational study

2020 ◽  
Vol 4 (20) ◽  
pp. 5133-5145
Author(s):  
Kentaro Yonekura ◽  
Shigeru Kusumoto ◽  
Ilseung Choi ◽  
Nobuaki Nakano ◽  
Asahi Ito ◽  
...  
Keyword(s):  
T Cell ◽  
Observational Study ◽  
Cytotoxic T Lymphocytes ◽  
Prospective Observational Study ◽  
Cell Subset ◽  
Cd8 T Cell ◽  
Lymphocyte Population ◽  
Cell Leukemia ◽  
Adult T Cell Leukemia ◽  
Multicenter Prospective Observational Study

Abstract Monitoring of Immune Responses Following Mogamulizumab-Containing Treatment in Patients with Adult T-Cell Leukemia-Lymphoma (ATL) (MIMOGA) is a multicenter prospective observational study to establish the most effective and safe treatment strategy using mogamulizumab for ATL patients (UMIN000008696). Mogamulizumab-naive patients were enrolled (n = 102), of whom 101 received mogamulizumab-containing treatment (68 acute, 18 lymphoma, 12 chronic, and 3 smoldering subtypes). At enrollment, there was a significant inverse correlation between serum soluble interleukin-2 receptor (sIL-2R) levels and percentages of Tax-specific cytotoxic T lymphocytes (Tax-CTLs) in the entire lymphocyte population or in the CD8+ T cell subset, but there was not a correlation with cytomegalovirus pp65–specific cytotoxic T lymphocytes (CMV-CTLs). The overall response rate was 65%, and median progression-free survival and overall survival (OS) were 7.4 and 16.0 months, respectively. A higher percentage of Tax-CTLs, but not CMV-CTLs, within the entire lymphocyte population or in the CD8+ T cell subset was significantly associated with longer survival. Multivariate analysis identified the clinical subtype (acute or lymphoma type), a higher sIL-2R level, and a lower percentage of CD2−CD19+ B cells in peripheral blood mononuclear cells as significant independent unfavorable prognostic factors for OS. This indicates that a higher percentage of B cells might reflect some aspect of a favorable immune status leading to a good outcome with mogamulizumab treatment. In conclusion, the MIMOGA study has demonstrated that mogamulizumab exerts clinically meaningful antitumor activity in ATL. The patient’s immunological status before mogamulizumab was significantly associated with treatment outcome. Further time series immunological analyses, in addition to comprehensive genomic analyses, are warranted.

Endoscope‐assisted transoral accessory parotid mass excision: Multicenter prospective observational study

2019 ◽  
Vol 130 (5) ◽  
pp. 1218-1226 ◽  
Author(s):  
Jin Pyeong Kim ◽  
Dong Kun Lee ◽  
Jeong Hwan Moon ◽  
Jun Sun Ryu ◽  
Seung Hoon Woo
Keyword(s):  
Observational Study ◽  
Prospective Observational Study ◽  
Multicenter Prospective Observational Study

scholarly journals Eye Tumors in Childhood as First Sign of Tumor Predisposition Syndromes: Insights from an Observational Study Conducted in Germany and Austria

Cancers ◽  
2021 ◽  
Vol 13 (8) ◽  
pp. 1876
Author(s):  
Madlen Reschke ◽  
Eva Biewald ◽  
Leo Bronstein ◽  
Ines B. Brecht ◽  
Sabine Dittner-Moormann ◽  
...  
Keyword(s):  
Optic Nerve ◽  
Observational Study ◽  
Ciliary Body ◽  
Prospective Observational Study ◽  
Genetic Data ◽  
Detailed History ◽  
Cancer Early Detection ◽  
Physical Examinations ◽  
Eye Tumors ◽  
Multicenter Prospective Observational Study

Retinoblastoma and other eye tumors in childhood are rare diseases. Many eye tumors are the first signs of a genetic tumor predisposition syndrome and the affected children carry a higher risk of developing other cancers later in life. Clinical and genetic data of all children with eye tumors diagnosed between 2013–2018 in Germany and Austria were collected in a multicenter prospective observational study. In five years, 300 children were recruited into the study: 287 with retinoblastoma, 7 uveal melanoma, 3 ciliary body medulloepithelioma, 2 retinal astrocytoma, 1 meningioma of the optic nerve extending into the eye. Heritable retinoblastoma was diagnosed in 44% of children with retinoblastoma. One child with meningioma of the optic nerve extending into the eye was diagnosed with neurofibromatosis 2. No pathogenic constitutional variant in DICER1 was detected in a child with medulloepithelioma while two children did not receive genetic analysis. Because of the known association with tumor predisposition syndromes, genetic counseling should be offered to all children with eye tumors. Children with a genetic predisposition to cancer should receive a tailored surveillance including detailed history, physical examinations and, if indicated, imaging to screen for other cancer. Early detection of cancers may reduce mortality.

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